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Chordoma (also known as notochordal sarcoma) is a rare type of primary bone cancer that is commonly found at the bottom of the spine (sacrum) and base of skull. Chordoma originates from remnants of embryonic notochord. It begins in the developing embryo, and when you are born, it may go away but some of the cells may remain and eventually become cancerous.
It is most frequently diagnosed in ages between 40 and 60 years old, however the disease can occur at any age group, and it is more common to men than women.
Chordoma is a slow-growing tumor, and the treatment is difficult because of the location of the disease which is near the nerves of the spinal cord.
Symptoms
Signs and symptoms of chondroma depend on size and location of the tumor, and the following symptoms may indicate the disease:
- Back pain
- Numbness or muscle weakness especially at the arms and legs
- Headaches
- Visual problem
- Nosebleeds
- Bowel incontinence and/or urinary incontinence.
If you have any of the following signs or symptoms, you should see the doctor.
Causes
The cause of the chordoma has not yet determined by the experts, but risk factors had been identified that increase the risk of having the disease.
Risk factors
These are factors to increase risk of having chordoma:
- Age. The disease commonly affects adult at age 40 – 60 years old but may also occur at any age.
- Gender. It is more commonly found in men than in women.
- Family History. The risk of chordoma is increased if a direct member of the family is diagnosed with the disease.
- Genetic condition. If children develop genetic condition such as tuberous sclerosis, then the child will have a high risk to develop chordoma.
Diagnosis
The following test could help confirm the diagnosis and the location of the tumor.
- Diagnostic Imaging Procedure – includes Computerized Tomography (CT), and Magnetic Resonance Imaging (MRI). This is to determine the location and extent of the cancer.
- Tissue Biopsy – A sample of suspicious tissue is removed for examination under a microscope in the laboratory to check for cancer. The biopsy result will also help determine the cell type involved and the treatment needed.
Treatment
The size and location are some of the factors which can affect the treatment options as well as the general health and personal choice of treatment.
The treatment options for chordoma in the sacral spine are as follows:
- Surgery: The aim of surgery is to remove all of the cancer as well as some healthy tissues surrounding it. It may be difficult to undergo the surgery in some cases if the tumor is located near nerves or blood vessels. If it is not possible to remove the entire cancer, then the surgeons may opt to remove as much of the cancer as they can.
- Radiation therapy: There is a use of high energy beams, such as X-rays to kill the cancer cells. These beams are targeted at specific points on the body while the patient is lying down on a table and a machine is moving around. It can be used in order to shrink the cancer cells which surgery is not an option, as well as destroy the cancer cells that are left after surgery has been performed. There is also another type of radiation treatment called proton therapy which uses a higher dosage of radiation as well as protection for the healthy tissues and may be a more effective of treatment of chordoma.
- Radiosurgery: This procedure uses multiple beams of radiation to destroy the cancer in specific areas. Although the beams are not very powerful, but due to the precision of the location, it will target the specific chordoma cells. This is a good option if surgery is not recommended.
- Targeted therapy: These drugs are used in order to focus on specific abnormalities that are in the cancer cells. This can be used for treatment of chordoma when it spreads to other parts of the body.
